摘要:

AbstractFreeze‐fracture replicas of normal and dystrophic C57BL mouse muscle and kidney were examined to see whether there was a deficit in plasmalemmal particles which others suggest is a feature of dystrophies. When compared with normal membranes there was an increase in the particle density in dystrophic extensor digitorum longus muscle, a decrease in dystrophic soleus muscle, and no change in dystrophic kidney. Therefore there was not a general deficit in intramembrane particles in this dystrophic tissue. Indirect evidence supported the hypothesis that abnormalities in dystrophic mouse muscles are caused by abnormal motor input. The density of indentations, parallel to the T‐tubule, on the flat surface of the terminal cisternae can be modulated by the motor nerve. Changes were found in identation density in dystrophic muscle which were similar to changes seen after transection of the spinal cord in the mid‐thoracic region. There were parallel changes in contractile properties and identation density in dystrophic f

ISSN:0148-639X    

DOI:10.1002/mus.880050603

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractTwo cases of unilateral calf enlargement following S1radiculopathy are reported. “True” muscular hypertrophy could be confirmed by computerized tomography of the muscle. Muscle biopsy disclosed that fiber hypertrophy exceeded grouped fiber atrophy. In one case both fiber types were involved, and in the other mostly type 1 fibers were affected. Electromyography revealed dense pseudomyotonic activity and fasciculations. These findings are discussed in view of the current literat

ISSN:0148-639X    

DOI:10.1002/mus.880050604

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractWe have recently reported on the isolation and characterization of sarcoplasmic reticulum (SR) from normal and dystrophic mice. These purified fractions were similar in functional characteristics. We now present an analysis of the lipids in our purified SR. The lipids were generally found to be similar. Most of the differences found between the two preparations were consistent with a somewhat greater surface membrane contamination in SR fractions from dystrophic mice. This was so with respect to cholesterol content and fatty acid composition. A small decrease, however, in content of phosphatidylcholine was observed in the dystrophic SR fractions. Fluorescence polarization studies using the probe 1,6‐diphenyl‐1,3,5‐hexatriene in sarcoplasmic reticulum membranes over the temperature range 3 to 38 C showed slightly greater anisotropy in the dystrophic fractions, which is also consistent with a greater contamination of this fraction by surface membrane elements (sarcolemma and transverse tu

ISSN:0148-639X    

DOI:10.1002/mus.880050605

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractGenetically homozygous line 413 dystrophic chickens were given twice‐daily intraperitoneal injections of solutions containing branched‐chain amino acids (BCAA‐leucine, valine, isoleucine) either alone or in combination; and their α‐ketoacid analogs (α‐ketoisocaproic and α‐ketoisovaleric acids). Another trial consisted of an amino acid mixture containing BCAA. Amino acid supplementation in each case significantly prolonged righting ability measured regularly by a standardized flip‐test procedure. Enhanced functional ability was not generally accompanied by a decrease in plasma creatine kinase activity. However, a measurable increase in the affected pectoralis major muscle mass and protein content (female chickens in particular) was found with BCAA therapy. Moreover, the increase in muscle bulk was attended in some cases by a reduction in the relative number of degenerating fibers quantitated microscopically. Contrariwise, the amino acid mixture caused a reduction in pectoralis muscle mass. It is concluded that parenteral BCAA therapy offers limited benefit in retarding dystrophic symptom

ISSN:0148-639X    

DOI:10.1002/mus.880050606

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractNineteen patients with chronic renal failure receiving intermittent hemodialysis had single fiber electromyography studies of their extensor digitorum communis muscles. Jitter values were abnormal, more so in men than in women. The jitter abnormalities tended to improve after intermittent hemodialysis treatments lasting longer than 1 year.

ISSN:0148-639X    

DOI:10.1002/mus.880050607

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractProteins of the whole muscle homogenates of the slow‐twitch soleus (SOL) and fast‐twitch extensor digitorum longus (EDL) of normal and dystrophic C57BL/6J mice at 4, 8, 12, and 32 weeks of age were resolved on polyacrylamide isoelectric focusing gels. Gels of the normal SOL proteins at all ages contained two bands specific to SOL and not represented in EDL. Gels of normal EDL contained three bands highly amplified in EDL but barely detectable in SOL. The distribution of proteins in dystrophic SOL was abnormal at all age groups studied due, in part, to a decrease in the proportion of SOL‐specific proteins relative to other proteins in the muscle. The distribution of proteins in dystrophic EDL appeared abnormal first at 12 weeks due to a decrease in the relative proportion of EDL‐amplified proteins. Due to these and other changes, at 32 weeks the dystrophic SOL and EDL were almost indistinguishable on the basis of their proteins' distri

ISSN:0148-639X    

DOI:10.1002/mus.880050608

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractUltrastructural T system abnormalities are common in mature skeletal fibers from dystrophic chickens, but little is known of their development. Muscle strips from dystrophic and normal chickens aged 1 day through 4 weeks were examined in ultrathin plastic sections. At hatching, triads were sparse, with no apparent abnormalities. At 1 week, triads were more common; in some dystrophic fibers the T tubules were swollen, ballooned beyond the triad, or assembled asymmetrically, with or without supernumerary (>2) junctional sarcoplasmic reticulum components. At 2 weeks, many dystrophic fibers also displayed degenerative changes, i.e., myofibrillar disintegration and cytoplasmic dispersion. At 4 weeks, when normal triad assembly was complete, T system abnormalities were widespread in dystrophic fibers. Fiber degeneration had progressed to breakdown and replacement. Thus, derangement of the T system (which parallels onset of muscle dysfunction) superimposed on triad assembly suggests that compromised excitation‐contraction coupling is an early event in the chicken dystroph

ISSN:0148-639X    

DOI:10.1002/mus.880050609

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractChronic progressive or relapsing inflammatory polyneuropathy (CRIP) can be treated effectively with immunosuppressive drugs and/or plasma exchange (PE). This report describes a patient who responded dramatically and reproducibly to PE during four successive relapses and remained in remission while on medical immunosuppression with azathioprine and corticosteroids. The clinical course was closely correlated with electrophysiologic parameters such as compound muscle action potential amplitudes and distal latencies. The purified lgG fraction of PE filtrate bound to rat and human sciatic nerve on immunohistochemistry. Furthermore, a similar staining pattern was achieved by purified Fab fragments, which was absent in all control preparations. These findings support the hypothesis that circulating plasma factors may play a role in CRIP and that one of these factors may be an IgG antibody to peripheral nerve components.

ISSN:0148-639X    

DOI:10.1002/mus.880050610

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

摘要:

AbstractThe hypothesis that the existence of a family of isoenzymes is at the basis of the heterogeneity of esterase activity among skeletal muscles from dystrophic Re 129J and control littermate mice was tested using isoelectrofocusing (IEF) in polyacrylamide gel. All muscles considered showed not only quantitative heterogeneity, as previously observed in this laboratory, but also qualitative differences with regard to their esterase isoenzymes. Differences in the number and the relative amounts of isoenzymatic bands were found between different muscles from both control and dystrophic animals. In most dystrophic muscles a new isoenzymatic band appeared at pH 6.6. Another band, identified at pH 5.12, was more pronounced in dystrophic than in control muscles. The specificity of these observations is indirectly supported by the fact that other tissue, such as heart, liver, and kidney, did not show the quantitative or qualitative abnormality present in the dystrophic skeletal muscles.

ISSN:0148-639X    

DOI:10.1002/mus.880050611

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY

ISSN:0148-639X    

DOI:10.1002/mus.880050612

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1982

数据来源: WILEY